Pediatric neurology最新文献

{"title":"Enteral Ketamine for Status Epilepticus in Children with Epilepsy","authors":"Laura DiDomenico MD, MS (Trainee Author) ,&nbsp;Lisa C. Garrity SM, PharmD, BCPS ,&nbsp;Kelsey E. Poisson MD ,&nbsp;Eileen Broomall MD (Senior Author)","doi":"10.1016/j.pediatrneurol.2024.05.006","DOIUrl":"10.1016/j.pediatrneurol.2024.05.006","url":null,"abstract":"<div><h3>Background</h3><p>Approximately 10% to 20% of children with epilepsy experience status epilepticus (SE), and children with seizure clustering are at higher risk. Ketamine is growing in use for SE. This study examines the efficacy and safety of enteral ketamine in the treatment of convulsive status epilepticus (CSE) characterized by refractory seizure clusters and nonconvulsive status epilepticus (NCSE) in children with epilepsy.</p></div><div><h3>Methods</h3><p>Patient charts were reviewed retrospectively. Children with epilepsy aged one to 21 years presenting in SE and treated with enteral ketamine between September 1, 2021 and September 1, 2022 at a pediatric tertiary care center were identified. Resolution or reduction in seizure frequency within 48 hours, clinical presentation, endotracheal intubation, hospitalization duration, side effects, and readmission were assessed.</p></div><div><h3>Results</h3><p>Nine patients aged two to 21 years were identified. Six patients presented in CSE characterized by recurrent seizures, and three patients presented in NCSE. Five patients had genetic epilepsies, including PCDH19- and MECP2-related epilepsy. Seven patients had resolution or reduction in seizures within 48 hours of ketamine initiation. Two patients were intubated. Hospitalization duration ranged from one to 34 days. Three patients reported side effects. Three patient readmissions with early ketamine treatment had equal or shorter hospitalizations.</p></div><div><h3>Conclusions</h3><p>Enteral ketamine may prove an effective, well-tolerated option for treatment of convulsive and nonconvulsive SE in children with epilepsy, including genetic epilepsies, and may prevent intubation and shorten hospitalization time.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141056167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intravenous Lacosamide Therapy for Pediatric Patients With Cluster Seizures","authors":"Ryuki Matsuura MD, PhD ,&nbsp;Shin-ichiro Hamano MD, PhD ,&nbsp;Kenjiro Kikuchi MD, PhD ,&nbsp;Rikako Takeda MD ,&nbsp;Hirokazu Takeuchi MD ,&nbsp;Yuko Hirata MD, PhD ,&nbsp;Reiko Koichihara MD ,&nbsp;Takehiro Niitsu MD ,&nbsp;Ikuya Ueta MD ,&nbsp;Akira Oka MD, PhD","doi":"10.1016/j.pediatrneurol.2024.05.003","DOIUrl":"10.1016/j.pediatrneurol.2024.05.003","url":null,"abstract":"<div><h3>Background</h3><p>Few studies have investigated intravenous lacosamide use to treat cluster seizures in pediatric patients. Therefore, we aimed to investigate the efficacy and safety of intravenous lacosamide therapy in pediatric patients with cluster seizures.</p></div><div><h3>Methods</h3><p>We retrospectively evaluated the efficacy and safety of intravenous lacosamide therapy in 25 pediatric patients with cluster seizures at Saitama Children's Medical Center between March 2019 and June 2023. Cluster seizures were defined as a single seizure of less than five minutes duration, repeated three or more times within 12 hours, with recovery of consciousness between seizures. Response was defined as seizure freedom for at least 12 hours after lacosamide infusion.</p></div><div><h3>Results</h3><p>The median age at onset of epilepsy was 1.5 (0.0 to 9.8) years. The median seizure frequency was 5 (3 to 20) times per 12 hours. The etiologies were remote (n = 17), acute (n = 4), and progressive (n = 4). The median age at which intravenous lacosamide therapy was administered was 4.2 (0.0 to 11.3) years. The median lacosamide dose was 2.6 (1.3 to 5.2) mg/kg. In total, 12 of 25 patients (48.0%) responded. Among patients treated with intravenous lacosamide as first-line therapy, nine of 17 (52.9%) had complete seizure remission. The frequency of complete seizure remission in patients with remote etiologies was 58.8% (10 of 17); among them, seven of 12 (58.3%) patients with structural abnormalities showed complete seizure remission. No adverse events were observed.</p></div><div><h3>Conclusions</h3><p>Intravenous lacosamide therapy is a potentially useful treatment option for cluster seizures in pediatric patients.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.8,"publicationDate":"2024-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141030018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advancing the Understanding of Vesicle-Associated Membrane Protein 1-Related Congenital Myasthenic Syndrome: Phenotypic Insights, Favorable Response to 3,4-Diaminopyridine, and Clinical Characterization of Five New Cases","authors":"Daniel Natera-de Benito ,&nbsp;Alessia Pugliese ,&nbsp;Kiran Polavarapu ,&nbsp;Velina Guergueltcheva ,&nbsp;Ivailo Tournev ,&nbsp;Albena Todorova ,&nbsp;Joana Afonso Ribeiro ,&nbsp;Daniel M. Fernández-Mayoralas ,&nbsp;Carlos Ortez ,&nbsp;Loreto Martorell ,&nbsp;Berta Estévez-Arias ,&nbsp;Leslie Matalonga ,&nbsp;Steven Laurie ,&nbsp;Cristina Jou ,&nbsp;Jarred Lau ,&nbsp;Rachel Thompson ,&nbsp;Xinming Shen ,&nbsp;Andrew G. Engel ,&nbsp;Andres Nascimento ,&nbsp;Hanns Lochmüller ,&nbsp;Duygu Selcen","doi":"10.1016/j.pediatrneurol.2024.04.027","DOIUrl":"10.1016/j.pediatrneurol.2024.04.027","url":null,"abstract":"<div><h3>Background</h3><p>Congenital myasthenic syndromes (CMS) are a group of inherited neuromuscular junction (NMJ) disorders arising from gene variants encoding diverse NMJ proteins. Recently, the <em>VAMP1</em> gene, responsible for encoding the vesicle-associated membrane protein 1 (VAMP1), has been associated with CMS.</p></div><div><h3>Methods</h3><p>This study presents a characterization of five new individuals with VAMP1-related CMS, providing insights into the phenotype.</p></div><div><h3>Results</h3><p>The individuals with VAMP1-related CMS exhibited early disease onset, presenting symptoms prenatally or during the neonatal period, alongside severe respiratory involvement and feeding difficulties. Generalized weakness at birth was a common feature, and none of the individuals achieved independent walking ability. Notably, all cases exhibited scoliosis. The clinical course remained stable, without typical exacerbations seen in other CMS types. The response to anticholinesterase inhibitors and salbutamol was only partial, but the addition of 3,4-diaminopyridine (3,4-DAP) led to significant and substantial improvements, suggesting therapeutic benefits of 3,4-DAP for managing VAMP1-related CMS symptoms. Noteworthy is the identification of the <em>VAMP1</em> (NM_014231.5): c.340delA; p.Ile114SerfsTer72 as a founder variant in the Iberian Peninsula and Latin America.</p></div><div><h3>Conclusions</h3><p>This study contributes valuable insights into VAMP1-related CMS, emphasizing their early onset, arthrogryposis, facial and generalized weakness, respiratory involvement, and feeding difficulties. Furthermore, the potential efficacy of 3,4-DAP as a useful therapeutic option warrants further exploration. The findings have implications for clinical management and genetic counseling in affected individuals. Additional research is necessary to elucidate the long-term outcomes of VAMP1-related CMS.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.8,"publicationDate":"2024-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141045924","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Efficacy of Noninvasive Ventilation in Patients Affected by Rett Syndrome With Hypoventilation","authors":"Francesca Peri MD ,&nbsp;Claudio Cherchi MD ,&nbsp;Maria Beatrice Chiarini Testa MD ,&nbsp;Martino Pavone MD ,&nbsp;Elisabetta Verrillo MD ,&nbsp;Renato Cutrera MD","doi":"10.1016/j.pediatrneurol.2024.05.005","DOIUrl":"10.1016/j.pediatrneurol.2024.05.005","url":null,"abstract":"<div><h3>Background</h3><p>Rett syndrome is a progressive neurological disorder associated to several comorbidities that contribute significantly to impair lung function. Respiratory morbidity represents a major cause of death in this population. Little is known about the benefit of noninvasive ventilation.</p></div><div><h3>Methods</h3><p>We retrospectively enrolled patients with Rett syndrome who underwent a pneumological evaluation combined with a cardiorespiratory polygraphy and/or a pulse oximetry and capnography from 2012 to 2022.</p></div><div><h3>Results</h3><p>Medical records of 11 patients with Rett syndrome, mean age 13 ± 6 years, were evaluated. Most patients presented with both epilepsy and scoliosis. Five patients showed a pathologic sleep study and/or impaired night gas exchange: mean obstructive apnea-hypopnea index was 4 ± 3 events/hour; mean and minimal SpO₂ were, respectively, 93% ± 2% and 83% ± 6%, while mean and maximal transcutaneous carbon dioxide monitoring (PtcCO₂) were, respectively, 51 ± 5 mm Hg and 55 ± 8 mm Hg; and mean oxygen desaturation index was 13 ± 11 events/hour. These patients started noninvasive ventilation with clinical benefit and improved gas exchange mostly in terms of PtcCO₂ (mean PtcCO₂ 51 ± 5 mm Hg before and 46 ± 6 mm Hg after noninvasive ventilation).</p></div><div><h3>Conclusions</h3><p>Noninvasive ventilation is a suitable option for patients with Rett syndrome.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141055332","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroimaging and Neurological Outcomes in Perinatal Arterial Ischemic Stroke: A Systematic Review and Meta-Analysis","authors":"Lisa Pabst MD ,&nbsp;Catherine R. Hoyt PhD, OTD ,&nbsp;Ryan J. Felling MD, PhD ,&nbsp;Alyssa E. Smith MD ,&nbsp;Karen Harpster PhD, OTR/L ,&nbsp;Andrea C. Pardo MD ,&nbsp;Jeffrey A. Bridge PhD ,&nbsp;Bin Jiang MD, PhD ,&nbsp;Alison Gehred BA ,&nbsp;Warren Lo MD","doi":"10.1016/j.pediatrneurol.2024.04.029","DOIUrl":"10.1016/j.pediatrneurol.2024.04.029","url":null,"abstract":"<div><h3>Background</h3><p>Prediction of outcomes in perinatal arterial ischemic stroke (PAIS) is challenging. We performed a systematic review and meta-analysis to determine whether infarct characteristics can predict outcomes in PAIS.</p></div><div><h3>Methods</h3><p>A systematic search was conducted using five databases in January 2023. Studies were included if the sample included children with neonatal or presumed PAIS; if infarct size, location, or laterality was indicated; and if at least one motor, cognitive, or language outcome was reported. The level of evidence and risk of bias were evaluated using the Risk of Bias in Non-Randomized Studies of Interventions tool. Meta-analyses were conducted comparing infarct size or location with neurological outcomes when at least three studies could be analyzed.</p></div><div><h3>Results</h3><p>Eighteen full-text articles were included in a systematic review with nine included in meta-analysis. Meta-analyses revealed that small strokes were associated with a lower risk of cerebral palsy/hemiplegia compared with large strokes (risk ratio [RR] = 0.263, <em>P</em> = 0.001) and a lower risk of epilepsy (RR = 0.182, <em>P</em> &lt; 0.001). Middle cerebral artery (MCA) infarcts were not associated with a significantly different risk of cerebral palsy/hemiplegia compared with non-MCA strokes (RR = 1.220, <em>P</em> = 0.337). Bilateral infarcts were associated with a 48% risk of cerebral palsy/hemiplegia, a 26% risk of epilepsy, and a 58% risk of cognitive impairment.</p></div><div><h3>Conclusions</h3><p>Larger stroke size was associated with worse outcomes across multiple domains. Widely heterogeneous reporting of infarct characteristics and outcomes limits the comparison of studies and the analysis of outcomes. More consistent reporting of infarct characteristics and outcomes will be important to advance research in this field.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.8,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141034737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Wall-Eyed Bilateral Internuclear Ophthalmoplegia as Part of the Clinical Presentation of Pediatric Multiple Sclerosis, Successfully Treated With Therapeutic Plasma Exchange","authors":"","doi":"10.1016/j.pediatrneurol.2024.05.002","DOIUrl":"10.1016/j.pediatrneurol.2024.05.002","url":null,"abstract":"","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141039161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bedtime Regularity and Sleep Sufficiency in Children With Tourette Syndrome","authors":"Valerie S. Swisher BS,&nbsp;Serene Liu BS,&nbsp;Emily J. Ricketts PhD","doi":"10.1016/j.pediatrneurol.2024.05.001","DOIUrl":"10.1016/j.pediatrneurol.2024.05.001","url":null,"abstract":"<div><h3>Background</h3><p>Despite research demonstrating sleep disturbance in children with Tourette syndrome (TS), few studies have examined bedtime regularity and sleep sufficiency, two important sleep health dimensions. Therefore, this study examined bedtime regularity and sleep sufficiency in children with TS relative to matched healthy control subjects, and its associated demographic, clinical, and behavioral factors.</p></div><div><h3>Methods</h3><p>Participants were 384 parents or caregivers of children aged three to 17 years, including 192 with current TS and 192 matched healthy control subjects drawn from the 2020-2021 cycle of the National Survey of Children's Health. Parents completed questions assessing demographic (i.e., age, race, sex), clinical (i.e., attention-deficit/hyperactivity disorder [ADHD], autism spectrum disorder, anxiety, depression, tic severity, behavioral or conduct problems, ADHD medication, health condition-related impairment), and behavioral (i.e., screen time) characteristics. Mann-Whitney U test and chi-square test of independence were performed to compare groups on bedtime regularity and sleep sufficiency, respectively. Ordinal regression and binary logistic regression without and with backward elimination were performed to evaluate indicators of bedtime regularity and sleep sufficiency, respectively, in children with TS.</p></div><div><h3>Results</h3><p>Children with current TS had significantly poorer bedtime regularity, but not sleep sufficiency, relative to matched healthy control subjects. In children with TS, anxiety and two or more hours of daily screen time were associated with higher likelihood of poor bedtime regularity. Autism was associated with lower likelihood of insufficient sleep, and depression was associated with increased likelihood of insufficient sleep.</p></div><div><h3>Conclusions</h3><p>Findings put forth screen time, anxiety, and depression as intervention targets to optimize sleep health in children with TS.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141024244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Two Cases of Pediatric Leucine-Rich Glioma-Inactivated Protein-1 Encephalitis: Clinical Course, Challenges, and Implications","authors":"Khushboo Verma MBBS,&nbsp;Duriel Hardy MD","doi":"10.1016/j.pediatrneurol.2024.04.031","DOIUrl":"10.1016/j.pediatrneurol.2024.04.031","url":null,"abstract":"<div><h3>Background</h3><p>Leucine-rich glioma-inactivated protein 1 (LGI-1) encephalitis is a rare form of autoimmune limbic encephalitis. Although relatively well documented in adults, pediatric cases are rare and remain poorly understood.</p></div><div><h3>Methods</h3><p>We reviewed two pediatric cases of LGI-1 encephalitis from a single tertiary care facility retrospectively. The detailed analysis included assessment of the initial presentation, clinical progression, diagnostic challenges, treatments, and outcome. To contextualize the differences between pediatric and adult manifestations of disease, we compared these findings with existing literature.</p></div><div><h3>Results</h3><p>Both cases illustrate the diagnostic challenges faced at initial presentation due to the rarity of this diagnosis in children and the absence of characteristic faciobrachial dystonic seizures, which is common in adults. The constellation of neuropsychiatric symptoms and refractory focal seizures led to a high clinical suspicion for autoimmune encephalitis, therefore, both cases were treated empirically with intravenous methylprednisolone. The diagnosis in both cases was confirmed with positive serum antibody testing, reinforcing that LGI-1 antibodies are more sensitive in the serum rather than the cerebrospinal fluid (CSF). Seizure control and improvement in cognitive symptoms was achieved through a combination of immunotherapy and antiseizure medications.</p></div><div><h3>Conclusions</h3><p>This case series underscores the significance of considering LGI-1 encephalitis in the differential diagnosis of pediatric patients exhibiting unexplained neuropsychiatric symptoms and focal seizures and emphasizes the importance of performing both serum and CSF antibody testing. It is necessary to conduct further research to identify the full range of pediatric presentations and to determine the optimal treatment protocol.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141023591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Spectrum of Intracranial Arteriopathies and Ischemic Strokes in Pediatric Tubercular Meningitis: A Tricentric Study From Eastern India","authors":"Dr. Suman Das MBBS, MD (Pediatric Medicine), DM (Neurology) (Assistant Professor) ,&nbsp;Dr. Biman Kanti Ray MBBS, MD (Medicine), DM (Neurology) (Professor) ,&nbsp;Dr. Madhumita Nandi MBBS, MD (Pediatric Medicine) (Professor) ,&nbsp;Dr. Gobinda Mondal MBBS, MD (Pediatric Medicine) (Associate Professor) ,&nbsp;Dr. Dilip Kumar Paul MBBS, MD (Pediatric Medicine) (Professor)","doi":"10.1016/j.pediatrneurol.2024.04.026","DOIUrl":"10.1016/j.pediatrneurol.2024.04.026","url":null,"abstract":"<div><h3>Introduction</h3><p>Addressing the need to uniformly classify arteriopathies among patients with arterial ischemic stroke (AIS) due to tubercular meningitis (TBM), we used the Childhood AIS Standardised Classification and Diagnostic Evaluation (CASCADE) criteria.</p></div><div><h3>Methods</h3><p>This tri-centric prospective study included children aged 0.5-12 years with TBM and AIS. Magnetic resonance angiographies (MRAs) were done during admission and repeated 3 and 12 months after discharge. Arteriopathies were classified according to the primary CASCADE criteria. We used the modified Pediatric Alberta Stroke Programme Early Computed Tomography Score as an ordinal measure of infarct volume. The severity of arteriopathies was graded using the focal cerebral arteriopathy severity score (FCASS). The final outcomes were measured at the 12-month follow-up visit using the Pediatric Stroke Outcome Measure (PSOM).</p></div><div><h3>Results</h3><p>Out of 55 patients, 64% had MRA-evidenced arteriopathies and 84% had multiple infarcts. The middle cerebral (46%) and internal carotid arteries (22%) were most commonly affected. The basal ganglia (70%) and the cerebral cortex (61%) were most commonly infarcted. CASCADE categories included 3b (40%), 1d (38%), 2b (16%), 2c (5%), progressive (32%), and stable (44%) arteriopathies. Younger age, hypertrophic pachymeningitis, cortical infarcts, recurrent strokes, progressive arteriopathies, EEG abnormalities, and mortality were significantly higher among patients with MRA-proven arteriopathies. Patients with progressive arteriopathies had a significantly higher prevalence of hypertrophic pachymeningitis, cortical infarcts, and recurrent strokes. FCASS correlated positively with outcomes measured by the Pediatric Stroke Outcome Measure and modified Pediatric Alberta Stroke Programme Early Computed Tomography Score.</p></div><div><h3>Conclusion</h3><p>The CASCADE classification clarified the arteriopathy patterns, enabling us to correlate them with the characteristics of the infarcts. FCASS is useful to grade the arteriopathy severity and progression in TBM.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.8,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141050027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric Palliative Epilepsy Surgery: A Report From the Pediatric Epilepsy Research Consortium (PERC) Surgery Database","authors":"Mary Jeno MD ,&nbsp;M. Bridget Zimmerman PhD ,&nbsp;Sabrina Shandley PhD ,&nbsp;Lily Wong-Kisiel MD ,&nbsp;Rani Kaur Singh MD ,&nbsp;Nancy McNamara MD ,&nbsp;Erin Fedak Romanowski DO ,&nbsp;Zachary M. Grinspan MD, MS ,&nbsp;Krista Eschbach MD ,&nbsp;Allyson Alexander MD ,&nbsp;Patricia McGoldrick NP, MPA, MSN ,&nbsp;Steven Wolf MD ,&nbsp;Srishti Nangia MD ,&nbsp;Jeffrey Bolton MD ,&nbsp;Joffre Olaya MD ,&nbsp;Daniel W. Shrey MD ,&nbsp;Samir Karia MD ,&nbsp;Cemal Karakas MD ,&nbsp;Priyamvada Tatachar MBBS, MD ,&nbsp;Adam P. Ostendorf MD ,&nbsp;Michael Ciliberto MD","doi":"10.1016/j.pediatrneurol.2024.04.028","DOIUrl":"10.1016/j.pediatrneurol.2024.04.028","url":null,"abstract":"<div><h3>Background</h3><p>Epilepsy surgery is an underutilized resource for children with drug-resistant epilepsy. Palliative and definitive surgical options can reduce seizure burden and improve quality of life. Palliative epilepsy surgery is often seen as a “last resort” compared to definitive surgical options. We compare patient characteristics between palliative and definitive epilepsy surgical patients and present palliative surgical outcomes from the Pediatric Epilepsy Research Consortium surgical database.</p></div><div><h3>Methods</h3><p>The Pediatric Epilepsy Research Consortium Epilepsy Surgery database is a prospective registry of patients aged 0-18 years undergoing evaluation for epilepsy surgery at 20 pediatric epilepsy centers. We included all children with completed surgical therapy characterized as definitive or palliative. Demographics, epilepsy type, age of onset, age at referral, etiology of epilepsy, treatment history, time-to-referral/evaluation, number of failed anti-seizure medications (ASMs), imaging results, type of surgery, and postoperative outcome were acquired.</p></div><div><h3>Results</h3><p>Six hundred forty patients undergoing epilepsy surgery were identified. Patients undergoing palliative procedures were younger at seizure onset (median: 2.1 vs 4 years, <em>P</em>= 0.0008), failed more ASM trials before referral for presurgical evaluation (<em>P</em>=&lt;0.0001), and had longer duration of epilepsy before referral for surgery (<em>P</em>=&lt;0.0001). During presurgical evaluation, patients undergoing palliative surgery had shorter median duration of video-EEG data collected (<em>P</em>=0.007) but number of cases where ictal data were acquired was similar between groups. The most commonly performed palliative procedure was corpus callosotmy (31%), followed by lobectomy (21%) and neuromodulation (82% responsive neurostimulation vs 18% deep brain stimulation). Palliative patients were further categorized into traditionally palliative procedures vs traditionally definitive procedures. The majority of palliative patients had 50% reduction or better in seizure burden. Seizure free outcomes were significantly higher among those with traditional definitive surgeries, 41% (95% confidence interval: 26% to 57%) compared with traditional palliative surgeries and 9% (95% confidence interval: 2% to 17%). Rate of seizure freedom was 46% at 24 months or greater of follow-up in the traditional definitive group.</p></div><div><h3>Conclusions</h3><p>Patients receiving palliative epilepsy surgery trialed more ASMs, were referred later after becoming drug resistant, and had longer gaps between drug resistance and epilepsy surgery compared with patients undergoing definitive epilepsy surgery. The extent of surgical evaluation is impacted if surgery is thought to be palliative. A majority of palliative surgery patients achieved &gt;50% seizure reduction at follow-up, both in groups that received traditionally palliative and tradition","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.8,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0887899424001577/pdfft?md5=559c4657a78b3f021038c359efa5d6cc&pid=1-s2.0-S0887899424001577-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141030429","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}